Atrioventricular (AV) septal defect (formerly called AV canal or endocardial cushion defect or primum atrial septal defect (ASD) with cleft mitral valve) may be either complete (single AV valve) or partial (2 well formed AV valves). Patients with complete AV septal defect rarely survive without operation for more than a few years. Patients with partial AV septal defect, however, commonly survive for many years but not as long as patients with secundum ASD. Prolonged survival with partial AV septal defect (primum ASD), however, is unusual, particularly in the presence of severe mitral regurgitation and pulmonary hypertension, but such was the case in a 59-year-old woman described in this report. This manuscript reviewed previously reported patients with partial AV septal defect show survival more than 50 years. This is the first report to describe mitral anular calcium in a patient with a primum type ASD, as well as, calcium in the pulmonary trunk. Thus, the patient described was unusual on 7 counts (prolonged survival, severe mitral regurgitation, severe pulmonary hypertension, mitral anular calcium, pulmonary truncal calcium, coronary arterial calcium and severe coronary atherosclerosis necessitating aorto-coronary bypass).